Pathology of the Vestibulocochlear Nerve

Joel D. Swartz, MD
KEYWORDS
 Internal auditory canal  Vestibulocochlear nerve  Hearing loss

This article covers congenital deformity of the internal auditory canal (IAC), neoplastic and pseudoneoplastic lesions with special detailed emphasis on schwannoma of the eight cranial nerve (acoustic neuroma), non-neoplastic IAC/cerebellopontine angle mass (CPA) pathology, including vascular loops and numerous additional differential diagnostic entities with particular emphasis on nonneoplastic meningeal disease.

CONGENITAL DEFORMITY OF THE INTERNAL AUDITORY CANAL

The caliber of the internal auditory canal (IAC) depends upon the development of the vestibulocochlear nerve.1 This is consistent with findings noted in the evaluation of the other skull base foramina, virtually all of which develop in response to the presence of the regional nerve or artery. As such, congenital vestibulocochlear nerve deficiency commonly is associated with a hypoplastic IAC containing only the facial nerve (Fig. 1). Although measurements are somewhat arbitrary, the IAC is considered hypoplastic when less than 2 mm in diameter. This usually is appreciated best upon examination in the axial and coronal plane; however, high-resolution thin-section oblique sagittal T2W MR imaging not only allows demonstration of this small canal but also depicts to best advantage absence/hypoplasia of the vestibulocochlear nerve (Fig. 2).2 The cochlear nerve is defined as hypoplastic when similar in size or smaller than either of the vestibular nerves. Cochlear nerve deficiency in the presence of a normal-sized internal auditory canal implies that it is acquired after formation of the IAC. This has been observed most commonly in patients who have labyrinthitis ossificans (Fig. 3).

Germantown Imaging Associates, Gladwyne, PA 19085, USA E-mail address: swartzjd@aol.com Neuroimag Clin N Am 18 (2008) 321346 doi:10.1016/j.nic.2008.02.001 1052-5149/08/$ see front matter 2008 Elsevier Inc. All rights reserved.

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Abnormality of vestibulocochlear nerve caliber has crucial implications for patients requiring cochlear implantation.3 Absence of the cochlear nerve is the only complete contraindication to this procedure. In addition to the IAC caliber, the imaging specialist must evaluate the status of the cochlear nerve aperture at the fundus of the IAC. Stenosis of this aperture consistently is associated with cochlear nerve deficiency also (Fig. 1B).4 Additional considerations regarding cochlear implantation are discussed later in this article. Narrowing of the IAC may occur secondary to exostoses or osteomas. These bony lesions are rare, but they may clinically mimic vestibular schwannomas by compression of the vestibulocochlear nerve. Hearing loss and vestibular symptoms including dizziness and vertigo may improve after surgical removal of the bony lesion. Compromise of the IAC also may occur as a complication of several of the otodystrophies, including Pagets disease and fibrous dysplasia. A large IAC (greater than 9mm) may be incidental. Bilateral IAC enlargement is seen with neurofibromatosis (NF)-2 secondary to bilateral acoustic tumors. NF also may result in IAC widening because of dural ectasia without neoplasm. The large IAC also has other important implications. Special attention to the fundus (lateralmost aspect) of the canal may reveal an unusually wide neural aperture leading to the cochlea or vestibule. This implies increased pressure within the perilymphatic space and predisposes to abnormal flow of perilymph (gusher) upon stapes manipulation. This can occur with various congenital inner ear malformations but is a classic manifestation of x-linked progressive mixed deafness (XLPMD) (Fig. 4).5 These male patients present with mixed hearing loss possibly masquerading

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Fig. 1. Hypoplastic internal auditory canal (IAC), occluded cochlear neural foramen. (A,B) Axial CT images of the left ear reveal an extremely narrow internal auditory canal (thick black arrow) contiguous with the anterior genu of the facial nerve canal (white arrow) with a bony plate occluding the cochlear neural foramen (long black arrow). (C) Sagittal T2WI of the left ear reveals a vacant anteroinferior fundal IAC quadrant (white arrow) indicating that the cochlear nerve is absent. (D) Sagittal T2WI of the right ear reveals a normal cochlear nerve (white arrow). (Courtesy of C.D. Phillips, MD, Charlottesville, VA.)

as otosclerosis clinically. If the deformity is recognized before middle ear exploration, the imaging specialist can provide a potentially life-saving service.

Schwannoma of the Eighth Cranial Nerve (Acoustic Neuroma)

Overview Schwannoma of the eighth cranial nerve (acoustic neuroma) is by far the most common CPA neoplasm.69 They are noncalcifying, slow-growing, well-encapsulated lesions of midlife that account for 6% to 10% of all intracranial tumors and 60% to 90% of CPA tumors. They are somewhat more common and tend to be larger in women. There are strong indications that pregnancy may accelerate the growth of this tumor. Schwannomas most commonly present as a combined IAC/CPA lesion; however, they may be entirely intracanalicular in nature. Interestingly, this latter subgroup of lesions is more common in men. Schwannomas arising from the extracanalicular portion of

NEOPLASTIC AND PSEUDONEOPLASTIC

The differential diagnosis of the IAC/cerebellopontine angle mass (CPA) is fairly clear-cut in that there is universal agreement regarding the four most common entities. Schwannoma of the eighth cranial nerve (acoustic neuroma) is the most common, followed by meningioma, arachnoid cyst, and epidermoid (in that order). The next several paragraphs summarize the most important clinical and imaging findings of these entities. Subsequent paragraphs will focus on the less common diagnostic entities.

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Fig. 2. Cochlear nerve aplasia. (A) Axial T2W drive image reveals that the cochlear nerve is absent (black arrow) from the anteroinferior quadrant of the internal auditory canal. The cochlear neural foramen is developmentally occluded (white arrow). (B) Sagittal T2W drive image of the left ear confirms the vacant anteroinferior quadrant (white arrow). (C) Sagittal T2W drive image of the right ear for comparison reveals a normal cochlear nerve (white arrow). (Courtesy of Jan W. Casselman, MD, PhD, Bruges, Belgium.)

the nerve may involve only the CPA cistern, sparing the IAC and masquerade as meningioma, metastasis, or exophytic brainstem tumor. Intralabyrinthine schwannomas are rare and will be discussed in detail later. Clinical issues Eighth nerve schwannomas typically present with progressive unilateral high-frequency retrocochlear sensorineural hearing loss, presumably resulting from compression/infiltration of auditory (cochlear) nerve fibers. This hearing loss is often manifested clinically by disproportionately deficient speech discrimination (compared with pure tone loss) as measured by brainstem electric response audiometry.8 Sudden worsening of hearing deficit occurs commonly (26%) and presumably is associated with occlusion of the internal auditory branch of the anterior inferior cerebellar artery (AICA). A small number of patients have normal hearing (or symmetric loss). The clinician must maintain suspicion when nonauditory symptoms predominate such as tinnitus or vertigo. Tinnitus in this context is typically high-pitched and unilateral (tumor side). Vertigo, an episodic illusion of motion, and horizontal nystagmus, are manifestations of peripheral vestibular dysfunctions that typically occur early in the course of tumor development, possibly because of disruption of vascular supply. Disequilibrium, a continuous sense of instability, occurs with larger lesions, possibly

because of deafferentation (compromise of sensory nerve impulses caused by injury of sensory nerve fibers). The presence of vertical nystagmus often implies a large tumor with brainstem compression. The facial nerve may undergo substantial torsion without losing its functional integrity; therefore associated facial palsy is unusual. Recall that although the facial nerve is predominately motor, there is a sensory component that supplies a portion of the external auditory canal and pinna. Diminished sensation in this distribution is referred to as Hitselbergers sign. Large lesions are more likely to cause fifth nerve symptoms (facial numbness, diminished corneal reflex). Facial pain, presumably caused by vascular impingement on the trigeminal root entry zone, is uncommon but not rare. Intention tremor and ataxia result from cerebellar compression. Despite the progressive nature of the hearing deficit, eighth nerve schwannomas arise from the vestibular rather than the cochlear portion of the nerve in approximately 85% of these patients. The superior and inferior vestibular nerves are equally likely to be the site of origin. Imaging issues The internal auditory canals have a strong tendency for symmetry, similar to the optic canals. This was the historical basis for schwannoma diagnosis with plain film and polytomography, modalities that required precise comparative

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Fig. 3. Acquired cochlear nerve atrophy. (A) Axial CT. The cochlea (large black arrow) and to a lesser degree, the vestibule (small black arrow) are ossified. Axial (B) and sagittal (C) T2W fast-spin-echo (FSE) images. A small caliber cochlear nerve (black arrowhead) is identified within the anteroinferior IAC quadrant. Incidental note is made of a vascular loop (white arrow). (D) Normal axial T2W FSE image for comparison reveals a normal cochlear nerve (white outlined arrow). (From Swartz JD, Harnsberger HR. Imaging of the temporal bone. New York: Thieme, Incorporated; 1998; with permission.)

measurements and identification of subtle erosive change. With the development of CT, direct visualization of the CPA component of the tumor could be accomplished, and it simultaneously was discovered that a significant percentage of normal individuals have asymmetric IACs. Despite advancements in CT, the direct diagnosis of intracanalicular lesions remained difficult until the development of gas CT cisternography. This technique required the placement of a small amount (2 to 3 mL) of intrathecal air or CO2 by means of lumbar puncture.9 The patient was imaged in the decubitus position with the affected ear up. Using this technique, very small lesions first were diagnosed, and an appreciation of CPA/IAC neurovascular anatomy was mandated. The emergence of

thin-section gadolinium-enhanced T1 weighted MR imaging sequences revolutionized diagnosis, as very small lesions easily could be diagnosed less invasively, and larger lesions were characterized more readily (Figs. 5 and 6). More recently, thin-section T2 weighted fast-spin-echo (FSE) and gradient-echo techniques have been developed that provide highly sensitive screening for these lesions without the use of gadolinium. FSE images have the advantage of far less tendency for magnetic susceptibility artifact, and resolution is increased further by the use of phased array receiver coils. Cerebrospinal fluid (CSF) provides an excellent contrast in this context, as virtually all schwannomas are of lower T2 signal intensity. This contrast is more apparent with longer TR

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Fig. 4. X-linked deafness. (A) Axial CT reveals patulous internal auditory canal (IAC) (*) and large defect at IAC fundus resulting in direct communication with the cochlear apex (white short arrow). (B) Axial T2WI reveals normal caliber neural structures and confirms wide communication (white long arrow). (Courtesy of Jan W. Casselman, MD, PhD, Bruges, Belgium.)

(4000 to 5000 ms). Such long repetition times (TR) are practical because of the decreased acquisition time resulting from the long echo trains inherent to this technique.10 There is some disagreement regarding the precise site of origination of eighth nerve schwannomas. It has been postulated that they arise in most cases at the transition zone between the myelin produced by the oligodendroglia (central myelin) and that produced by Schwanns cells (peripheral myelin). This neuroglial/neurilemmal junction, referred to as the Obersteiner-Redlich zone, occurs most commonly at the Scarpas (vestibular) ganglion typically (but not invariably) located at or in close proximity to the meatus (porus) of the IAC. Others have suggested that these tumors originate from Scarpas ganglion because this is the area of the highest concentration of

Schwann cells.11 Extracanalicular lesions invariably arise in close proximity to the meatus. No vestibular Schwannoma ever has been diagnosed at the brainstem root entry zone or even within the medial half of the cisternal segment of the nerve. Schwannomas only uncommonly arise within the lateral IAC (fundus), and therefore, imaging diagnosis of intracanalicular lesions in this specific location must be viewed with suspicion (Fig. 7). False-positive examinations are associated with the presence of relatively faint fundal enhancement. Nonneoplastic conditions encountered during surgical exploration of these lesions has lead to speculation that perhaps a wait-and-see approach should be advocated in this circumstance, with reimaging in 6 months. Disappearance of this faint enhancement has been documented in several patients.

Fig. 5. 8th nerve schwanoma with corresponding drawing. (A) Axial T1W postcontrast image reveals an intensely enhancing cerebellopontine angle mass (CPA) mass. (B) Corresponding drawing depicts a mass emanating from the internal auditory canal, bulging into CPA and impinging upon the middle cerebellar peduncle. (Courtesy of Amirsys, Inc., Salt Lake City, UT; with permission.)

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Fig. 6. 8th nerve schwannoma. (A) Axial T1W image. There is an isointense intracanalicular expansion (black arrow) bulging into the cerebellopontine angle mass. (B) Axial T1W image after contrast reveals intense homogeneous enhancement (outlined black arrow). (C) Axial T2W fast-spin-echo image reveals that the lesion extends throughout the fundus (outlined white arrow). (Courtesy of C.D. Phillips, MD, Charlottesville, VA.)

Histologic examination of schwannomas reveal compact Antoni type A tissue and loose textured, often cystic, Antoni type B tissue. The predominance of the Antoni type A variety helps explain this tendency toward T2 hyposignal relative to CSF. As schwannomas enlarge, regions of internal necrosis/cyst formation may result in a heterogeneous appearance. These are of higher signal intensity than CSF presumably because of the presence of hemorrhagic byproducts, necrotic material, or colloid-rich fluid. These larger lesions have a predominance of Antoni type B cells. In addition to intratumoral cysts, extramural (arachnoid) cysts also are associated with large lesions presumably secondary to elevation and deformation of the leptomeninges, which results in the formation of peritumoral adhesions, thereby creating a pseudoduplication of the arachnoid, trapping fluid between the leptomeninges and the mass.12,13 Extramural cysts also are associated with meningiomas by means of a similar mechanism. Intratumoral hemorrhage is rare and associated with head injury or vigorous physical exertion. Both intratumoral hemorrhage and cystic expansion may result in a rapid increase in tumor

volume. These patients often develop a sudden severe worsening of symptomatology (Fig. 8). The reader should be aware that diminished signal intensity in the vestibule on T2-weighted images has been demonstrated in patients who have cerebellopontine angle/IAC schwannoma (acoustic neurinoma), but not in patients who have CPA meningioma. This decreased signal intensity may result from increased protein concentration in the perilymph associated with acoustic tumors.14 Some patients who have vestibular schwannoma larger than 2 cm in diameter demonstrate a tiny area of hyperintensity in the dorsal brain stem on T2 weighted images at the lateral angle of the fourth ventricle floor. This may represent degeneration of the vestibular nucleus. This should not be confused with infarction.15 Eighth nerve schwannomas are typically benign and slow growing. Malignant degeneration is rare and usually associated with NF 1. These patients have an increased incidence of schwannomas; however, fewer than 5% have eighth nerve schwannoma. By contrast, the identification of bilateral eighth nerve schwannomas is the cornerstone of imaging diagnosis of the far less common

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Fig.7. 8th schwannoma of the inferior vestibular nerve. Axial T2W image (A) and axial postcontrast T1W image (B) reveals a lesion (white arrows) within the posteroinferior internal auditory canal quadrant at the level of the fundus. (Courtesy of Amirsys, Inc., Salt Lake City, UT; with permission.)

NF 2, so-called central NF (Fig. 9). These patients typically also have multiple meningiomas, neurofibromas, and glial tumors. Eighth nerve lesions may develop at a young age and MR imaging screening is recommended.9 The gene responsible for this neoplasm has been isolated to the long arm of chromosome 22. Surgical issues The collaboration of the imaging specialist and the surgical team is required in the choice of a definitive surgical procedure when an eighth nerve tumor is diagnosed. Three major approaches are

employed: translabyrinthine (TL), retrosigmoid (RS), and middle fossa (MF). Factors influencing the decision include the depth of tumor extension into the lateralmost IAC (fundal involvement), the size of a CPA component, and the prognosis for hearing conservation.9,16 If there is no serviceable hearing, or the prognosis for acceptable hearing is dim, the TL approach is most desirable, as surgical morbidity is reduced greatly. The TL approach is the most direct and allows for identification of the facial nerve and easy access to the fundus; it is required when an intralabyrinthine component is present. Despite

Fig. 8. 8th nerve schwannoma, hemorrhagic. (A) Axial T1W image, noncontrast: cerebellopontine angle mass mass with an area of hypersignal indicating the presence of methemoglobin (thin black arrows). (B) Axial T1W image, postcontrast reveals extent of lesion (thick black arrows). (Courtesy of Amirsys, Inc., Salt Lake City, UT; with permission.)

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CSF leak can occur with any of the three surgical approaches, although this complication is more common with the retrosigmoid approach and with larger tumors. Translabyrinthine surgery can result in a middle ear CSF fistula that drains through the nose through the eustachian tube, resulting in rhinorrhea. Loss of inner ear signal on T2WI correlates with loss of hearing in patients who have undergone surgery for CPA and IAC vestibular schwannoma, perhaps reflecting developing inner ear fibrous changes or ossification.17 Normal cochlear T2 signal is associated with hearing preservation in this circumstance. Radiosurgery (gamma knife treatment [GKT]) may be recommended, and the imaging specialist should be familiar with this procedure and the post-treatment appearance.18 This method has been helpful for patients who for other health reasons might be considered poor operative candidates. Often, there is paradoxically more heterogeneous enhancement in the first 6 to 12 months after GKT, as the center of the lesion undergoes cell death and fibrosis. Temporary enlargement has been reported within 2 years following treatment; therefore lesions larger than 3 cm and those with significant brainstem compression are excluded from radiosurgery. Postoperative imaging The imaging diagnosis of recurrent schwannoma may be challenging. Postoperative intracanalicular enhancement with gadolinium is very common. Such enhancement may be caused by recurrent tumor, adhesions, or meningeal irritation. Temporalis muscle and fascia, commonly used to seal the bony IAC defect created with the MF approach, results in a globular enhancement pattern. A linear enhancement pattern within the postoperative IAC is another predictable postoperative appearance.1922 Other postoperative features include T1 shortening in the labyrinth, possibly representing methemoglobin or elevated protein concentration, and the presence of new labyrinthine enhancement, which may be caused by labyrinthitis or granulation tissue. Intracanalicular enhancement also may occur within the facial nerve as the result of surgical manipulation of this structure and must not be confused with tumor recurrence. Postoperative MR imaging study performed within 3 days after surgery may be the most helpful asset for determining the presence of residual disease and serves as a baseline study for subsequent evaluation for recurrence. Follow-up studies that demonstrate a change in the enhancement pattern should be viewed with suspicion,

Fig. 9. Neurofibromatosis 2, bilateral vestibular schwannomas. Enhanced axial T1W image reveals bilateral intensely enhancing cerebellopontine angle mass/ internal auditory canal lesions. The right-sided lesion is larger, and there is shift of the fourth ventricle to the left. The left cavernous sinus is also abnormal (white arrow). (Courtesy of C.D. Phillips, MD, Charlottesville, VA.)

common misconception, the surgical exposure necessary for removal of large CPA components can be accomplished in experienced hands. Hearing conservation requires either an MF or RS approach. In this context, the imaging findings become especially crucial. The presence of a significant CPA component (greater than 1.5 cm) precludes the MF technique because of inadequate surgical exposure. The presence of a fundal component precludes the RS technique, because a portion of the labyrinth would have to be removed to expose the lateral third of the IAC (see Fig. 6). There are strong indications that FSE magnetic resonance sequences using T2 weighting are more sensitive to the presence of fundal tumor, because bright CSF provides better contrast than the dark CSF visualized on gadolinium MR imaging (T1 weighting). The MF approach is ideal for fundus lesions. The MF technique affords the greatest likelihood of hearing conservation but does so at the expense of the need for greater facial nerve manipulation, as the anterosuperior location of the nerve within the IAC places the nerve between the surgeon and the tumor. This is especially true when the neoplasm arises from the inferior (IVN) instead of the superior vestibular nerve (SVN). The RS approach is another example of potential hearing preservation surgery and affords the exposure to remove lesions with large CPA components. A subocciptal craniotomy medial to the sigmoid sinus is followed by cerebellar retraction and removal of the posterior lip of the IAC.

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especially when this enhancement becomes nodular.23 The retrosigmoid approach has the greatest risk of unintentional residual tumor in the lateral (fundal) aspect of the IAC, and this area should be searched carefully with postoperative scans.24 Intralabyrinthine schwannoma Intralabyrinthine schwannomas (ILS) arise from distal branches of the cochlear, superior vestibular, or inferior vestibular nerves. They may originate from vestibular nerve fibers of any of the cristae or maculae within the vestibule or nerve fibers of the cochlear nerve within the cochlea Box 1. ILS is rare, although recently a larger series and a more frequent occurrence has been reported.25 They are somewhat more common in patients who have NF-2, but most cases are sporadic.26 Lesions that are limited to the labyrinth are classified as intracochlear, intravestibular, and intravestibulocochlear. Lesions that also involve the IAC are described as transmodiolar (cochlea and IAC) or transmacular (vestibule and IAC) (Figs. 10 and 11). Lesions that also involve the middle ear are described as transotic (ME, labyrinth, and IAC) or tympanolabyrinthine (ME and labyrinth). All patients report sensorineural hearing loss that is of sudden onset in perhaps one third of cases. ILS is histologically identical to IAC counterparts and occasionally presents with Meniere-type symptoms, although recent data indicate that presentation is typically indistinguishable from that which occurs with typical eighth nerve schwannoma. A loss of conductive hearing is not uncommon and results from blockage of sound wave movement through inner ear fluid.
Box 1 Intralabyrinthine schwannoma Localized to labyrinth Intracochlear Intravestibular Intravestibulocochlear Internal auditory canal involvement Transmodiolar (cochlea and IAC) Transmacular (vestibule and IAC) Middle ear involvement Transotic (ME, labyrinth and IAC) Tympanolabyrinthine (ME and labyrinth)
Data from Tieleman A, Casselman JW, Somers TH, et al. Imaging of intralabyrinthine schwannomas: a retrospective study of 52 cases with emphasis on lesion growth. AJNR Am J Neuroradiol 2008 [Epub ahead of print].

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Fig. 10. Transmodiolar schwannoma. Axial enhanced T1W image reveals an enhancing lesion within the fundus of the internal auditory canal extending into the cochlea (white arrow). (From Swartz JD. Lesions of the cerebellopontine angle and internal auditory canal: diagnosis and differential diagnosis. Semin Ultrasound CT MR 2004;25(4):33252; with permission.)

Prior to the development of modern imaging methods, diagnosis usually was made during surgical labyrinthine ablation. Currently, the mainstays of diagnosis are thin-section Gadoliniumenhanced T1 weighted images that demonstrate a focal area of intense localized enhancement within the labyrinth (Fig. 12).27 This appearance is usually quite different from the faint, diffuse enhancement seen with labyrinthitis. Most cases will be discovered during routine evaluation of the rule out acoustic patient. The observer is encouraged to avoid IAC tunnel vision and meticulously examine the fluid-filled spaces of the labyrinth also. ILS also can be visualized with carefully preformed thin-section FSE T2WI as regions of hyposignal within the normally fluid intensity (bright) labyrinth (see Figs. 11 and 12). The reader should be aware that nontumorous labyrinthine enhancement may occur secondary to a fundal IAC lesion likely caused by venous engorgement and inflammation. This is referred to as IAC block.26 Intralabyrinthine neoplasms other than schwannoma are even more rare. Intralabyrinthine meningiomas are also intensely enhancing lesions.28,29 IAC meningioma likely arises from arachnoid granulations located along the dural lining of the neural foramina.30 They may spread to the labyrinth, resulting in intense enhancement, often with ossification and demineralization reminiscent of otospongiosis. The presence of associated ossification may be the key to diagnosis, although an atypically positioned ossified hemangioma also must be considered as a differential diagnostic possibility under this circumstance. Intravestibular lipomas have been reported manifest by precontrast T1 hypersignal, which disappears with fat suppression techniques. Neural

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Fig. 11. Cochleovestibular schwannoma. (A) Axial T2W image reveals abnormal hyposignal throughout the cochlear apex (outlined white arrow) and a portion of the vestibule (white arrow). (B) Axial contrast-enhanced T1W image reveals abnormal enhancement throughout the cochlear apex (outlined white arrow) and a portion of the vestibule (white arrow). (Courtesy of Jan W. Casselman, MD, PhD, Bruges, Belgium.)

transport of the meninx primitiva along the developing eighth nerve has been proposed as an etiology.31

Other Cerebellopontine Angle Mass Schwannomas

Vestibular schwannoma is clearly the most common intracranial nerve sheath tumor; however, schwannomas arising from CN5 to CN12 may result in a CPA mass. The nerve of origin of most CPA schwannomas often can be determined by the clinical symptomatology and specific imaging epicenter. The facial nerve schwannoma (FS) arising in the IAC or CPA may cause the most difficulty with differentiation from vestibular schwannoma.32

The key differentiating feature is the presence of an enhancing component extending along the labyrinthine segment of the facial nerve to the geniculate ganglion.33 At CT, bone windows may reveal enlargement of the facial nerve canal.

Meningiomas
Meningiomas are extra-axial neoplastic lesions arising from arachnoidal cap cells.7 Approximately 10% of all intracranial meningiomas arise in the posterior fossa. They are the second most common CPA tumor, constituting approximately 10% of masses in this location. CPA meningiomas usually arise from the posterior petrous surface or the underside of the tentorium. Intense enhancement

Fig.12. Intralabyrinthine schwannoma in the cochlear apex. Axial T2W fast-spin-echo image (A) and axial postcontrast T1W image (B) reveal a mass (white arrow) at the cochlear apex that enhances on the contrast enhanced image. (Courtesy of Amirsys, Inc., Salt Lake City, UT; with permission.)

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is the rule. Relative T2 hyposignal is characteristic of many of these lesions, especially the transitional and fibroblastic varieties (Fig. 13).9 Differentiation from acoustic tumor is based primarily on remoteness of the epicenter from the porus of the IAC and the presence of a broad base. Occasionally, meningiomas will masquerade as acoustic tumors when the epicenter is indeed at the meatus. Extension of tumor into the IAC is not rare in this context. The presence of a dural tail once was thought to be pathognomonic of meningioma and represent tumor extending along the adjacent dura; however, not only have histologic studies proven that the tail is often merely an inflammatory response, but numerous neoplasms and even non-neoplastic entities have been discovered to exhibit this nonspecific imaging characteristic.3437 Nonetheless, in some circumstances, this remains a somewhat useful imaging finding. Up to 75% of CPA meningiomas present with hearing loss similar to acoustic tumors.36 These tumors tend to present in middle-aged to elderly patients, especially women, and they are rare in young patients in the absence of NF2. As with meningiomas elsewhere, there is a tendency toward multiplicity, and the current and follow-up scans should be evaluated carefully for additional lesions. As with meningiomas found elsewhere, they tend to be hyperdense to brain at noncontrast CT imaging with homogeneous contrast enhancement. Focal areas of calcification are appreciated best before contrast and evident in up to 25% of cases. In contradistinction to schwannoma, necrosis and cystic degeneration are uncommon with meningiomas. Rarely, meningiomas may originate within the internal auditory canal, or even within the labyrinth. In these latter circumstances, differentiation from schwannoma is even more

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Fig.13. Meningioma. (A) Axial enhanced T1W image. (B) Axial T2W image. (C) Artist rendering. There is a homogeneously enhancing cerebellopontine angle mass mass (A) with a broad base and epicenter along the posterior petrous surface. The lesion is hypointense on the T2W image (B), characteristic of meninigioma (see text). There is fourth ventricle displacement confirming mass effect. Artist rendering (C) indicates a broad-base lesion that does not emanate from the IAC. (Courtesy of Amirsys, Inc., Salt Lake City, UT; with permission.)

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difficult. IAC meningiomas may spread to the labyrinth, often with ossification and demineralization reminiscent of otospongiosis. Relative T2 hypointensity is a helpful imaging finding, suggesting dense cellularity, and supports a diagnosis of tumor rather than reactive enhancement.3840

Box 2 Imaging epidermoid and arachnoid cyst Epidermoid Internal septations Lamellated appearance Encase Insinuate Higher signal than csf on flair Diffusion weighted imaging (DWI): restricted diffusion-marked hyperintensity Arachnoid cyst Homogeneous Smooth contour Displace Expand Identical to CSF on flair DWI: unrestricted diffusionidentical to CSF

Epidermoid
An epidermoid is an ectodermal inclusion that develops during neural tube closure in the third to fifth week of embryogenesis and comprises .2% to 1.8% of primary intracranial masses. Histologically identical to congenital cholesteatoma, they consist of a sac of exfoliated keratin lined by stratified squamous epithelium. They grow very slowly by means of desquamation and therefore often do not present until adulthood despite their congenital etiology. Epidermoids may become quite large before becoming symptomatic, because they have a strong tendency to grow along paths of least resistance, and, as such, conform to the surface of the brain tending to surround (encase) normal neural and vascular structures rather than displace or invade them. This tendency also often makes complete surgical resection difficult.41,42 The CPA is the most common location for the intracranial epidermoid (40% to 50%). They are the third most common overall CPA/IAC mass (3% to 7%) (after acoustic schwannoma and meningioma). At CT, most epidermoids are CSF density and do not enhance. Calcification is present in 10% to 25% of cases. An occasional lesion will have a faintly enhancing border or flecks of calcification. Most epidermoids are isointense to CSF on both T1 and T2 weighted magnetic resonance images. They exhibit this signal, because the cholesterol in epidermoid tumors is in the solid/crystalline state rather than the liquid state. The latter would result in bright T1 signal. The adamantinomatous craniopharyngioma typically contains this type of cholesterol; epidermoids rarely do.41 Most epidermoid cysts do not enhance, although some minimal rim enhancement occurs in approximately 25% of cases. Malignant transformation of an epidermoid (squamous cell carcinoma) is rare but should be considered if follow-up scans demonstrate a significant increase in the amount of enhancement (Box 2). Often, MR imaging is focused on differentiation from arachnoid cyst. Careful MR imaging examination commonly will reveal multiple internal septations within the epidermoid that are not present with arachnoid cyst. Furthermore, many epidermoids often have a lamellated appearance because of surface desquamation. This lamellation

as well as the tendency to have internal septations and to insinuate rather than displace are far more characteristic of epidermoid. They do not suppress completely on fluid-attenuated inversion recovery imaging and therefore are higher in signal intensity than CSF.43 State-of-the-art differentiation of epidermoids from arachnoid cysts requires DWI (Fig. 14). Epidermoids are bright on diffusion imaging because of low water diffusability (low apparent diffusion coefficient, or ADC). This restricted diffusion results in marked hyperintensity, quite different from arachnoid cysts.44 DWI signal also is useful for detecting postoperative residual epidermoid.45 The rare white epidermoid is an interesting variant that is hyperdense at CT, hyperintense on T1WI, and hypointense on T2W.46 This may be explained by high protein content, hemorrhage or the presence of saponified keratinized debris, and as indicated previously, cholesterol in the liquid state. Like epidermoid cysts, dermoid cysts are congenital ectodermal inclusions but are extremely rare, far less common than epidermoid cysts. Growth can lead to rupture of the cyst, causing a chemical meningitis. A common misconception is that dermoid cysts arise from both ectodermal and mesodermal elements. Their origin is entirely ectodermal. Imaging findings vary. Unruptured cysts are hyperintense, similar to fat on T1 weighted images. The masses have heterogeneous signal intensity on T2-weighted magnetic resonance images and vary from hypo- to hyperintense. Fatlike leptomeningeal and intraventricular deposits are diagnostic of a ruptured dermoid cyst.47

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Fig. 14. Epidermoid. (A) Axial T2W image. (B) Axial T1W image. (C) Axial diffusion image. (D) Artist rendering. There is a homogeneous mass in the left cerebellopontine angle mass, isointense with cerebrospinal fluid (A,B) and with fourth ventricle displacement. There is restricted diffusion (C). (Courtesy of C.D. Phillips, MD, Charlottesville, VA.)

Arachnoid Cysts
Arachnoid cysts are benign space-occupying lesions containing CSF. They reside entirely within the subarachnoid space but are surrounded by a thin membrane. They are typically unilocular, smoothly marginated, and expansile; they do not communicate with the ventricular system. The precise mechanism for arachnoid cyst development is unknown. These lesions may develop through failure of embryonic meninges to merge or they may occur secondary to splitting of the developing arachnoid. Arachnoid cysts are generally stable over time.48 The cyst wall is lined by flattened arachnoid cells rather than epithelium. Most arachnoid cysts are supratentorial. Fifty percent to 60% are found in the middle cranial fossa, anterior to the temporal lobes. Approximately 10% occur in the posterior fossa, most commonly in the CPA.

Arachnoid cysts are well demarcated extra-axial lesions that can displace adjacent structures. There is no identifiable internal architecture. They are isodense to CSF at CT and isointense to CSF on all magnetic resonance sequences. They do not enhance with either modality (Fig. 15). Occasionally, hemorrhage, high protein content, or lack of flow within the cyst may complicate the magnetic resonance appearance. As discussed previously, the most difficult lesion to distinguish from the arachnoid cyst is epidermoid.

Meningeal Metastases
Cerebellopontine angle metastatic disease can present as a distinct extra-axial mass mimicking schwannoma/meningioma or as meningeal metastases associated with diffuse seeding of the CSF (Fig. 16). Meningeal metastases is referred to by several ambiguous terms including

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Fig.15. Arachnoid cyst. (A) Axial T2W image. (B) Axial enhanced T1W image. (C) Artists rendering. There is a homogeneous fluid intensity nonenhancing right cerebellopontine angle mass mass (black arrow), compatible with an arachnoid cyst. The diffusion images were negative (unrestricted diffusion). Drawing illustrates that these lesions displace rather than encase the neural structures. (Courtesy of Amirsys, Inc., Salt Lake City, UT; with permission.)

leptomeningeal carcinomatosis, meningeal carcinomatosis, and carcinomatous meningitis.9 These terms are inexact, as these neoplasms are not always carcinomas. Additionally, they often involve

the pachymeninges and the leptomeninges and may not contain an inflammatory (-itis) component.49 Meningeal metastases from solid tumors (ie, breast) and lymphoproliferative malignancy

Fig.16. Meningeal metastases. (A) Axial unenhanced T1W image. (B) Axial enhanced T1W image. There is diffuse enhancement within the internal auditory canal bilaterally (black outlined arrows). In addition, there is diffuse thickening and enhancement of the leptomeninges (black arrows) and pachymeninges (white arrow).

Pathology of the Vestibulocochlear Nerve

(ie, lymphoma) involve both the pachymeninges and the leptomeninges. Seeding from primary central nervous system (CNS) tumors involve only the leptomeninges. Leptomeningeal neoplasm may be diffuse or discrete and nodular. Nodular disease may masquerade as a primary neoplasm such as schwannoma or meningioma. Involvement of multiple cranial nerves is common. In this context, both facial nerve dysfunction and vertigo/hearing loss may result from CPA/IAC dissemination. A new diagnosis of NF2 in an adult patient should be considered suspect even when bilateral CPA/IAC lesions are diagnosed, and a thorough search should be performed for other intracranial metastases and the CSF sampled for malignant cells. Metastatic melanoma has a predilection for the leptomeninges and as such distinctively involve the IAC/CPA.50 Amelanotic lesions appear identical to schwannoma; however, melanotic lesions are hyperintense on precontrast T1WI, resulting in a confusing imaging picture suggestive of lipomatous infiltratration. There is no fat suppression, which aids in diagnosis. inclusions, distinguishing them from epidermoid and dermoid, which are ectodermal in nature. They may be found in the CPA, IAC, or even more rarely, within the vestibule. Intracanalicular lipomas, despite their rarity, mandate routine precontrast thin-section T1 weighted images as part of a complete IAC examination.51 At CT, lipomas are of fatty density and do not enhance. At MR imaging, they follow the signal intensity of fat, with suppression of signal on fat saturation images (Fig. 17). CPA lipomas may present with symptoms related to cranial nerve irritation such as trigeminal neuralgia (CN5) or hemifacial spasm (CN7). Most commonly, they present with vertigo and hearing loss (CN8). Conservative management usually is preferred unless the symptoms are disabling, as neurovascular structures often pass through or are adherent to the lesion. Noncontrast T1 hypersignal also could result from a dermoid; in particular, ruptured dermoid must be considered in the differential diagnosis if CPA/IAC hypersignal is accompanied by numerous other subarachnoid hyperintense deposits. Retained pantopaque (iophendylate) also has signal characteristics identical to fat. T1 hypersignal within the IAC masquerading as lipoma has been described. Clinical history of previous myelogram is needed to suggest this diagnosis.9

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Lipoma and Mimics

Lipomas are composed of mature adipose tissue, which is thought to arise from aberrant differentiation of the meninx primitiva. They are mesodermal

Fig. 17. Lipoma, intracanalicular. Axial (A) and coronal T1W image (B) reveal a hyperintense intracanalicular expansion. (C) Axial T1W fat-suppressed image confirms the diagnosis of lipoma. (Courtesy of C.D. Phillips, MD, Charlottesville, VA.)

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Hamartomas of the IAC also have a tendency for adherence to neurovascular structures, making resection technically difficult.52 Lipochoristomas are rare tumors of the acoustic nerve containing mature adipose tissue mixed with varied amounts of fibrous tissue, vessels, and muscle fibers.53 type 1 loops (within the CPA), type 2 loops (at the porus extending up to 50% of IAC), and type 3 loops (extending beyond 50% of IAC). These authors found a statistically significant association with unilateral hearing loss and type 2 and 3 AICA loops. Other studies, however, have been less supportive of surgical intervention. These have indicated that radiologic demonstration of contact between a vascular loop formed by the AICA and the eighth cranial nerve on MR imaging scans should be considered a normal anatomic finding and should not, on its own, be used to support the diagnosis of a vascular compression syndrome. Clinical correlation is needed, and a cause and effect may be difficult to establish (Fig. 18). I would certainly advise that all vascular structures within the CPA/IAC be reported to the referring physician. Aneurysms Aneurysms localized to the CPA/IAC may arise from the AICA.58,59 They are rare and may be misinterpreted as vestibular schwannoma (Fig. 19). They may present with headache (subarachnoid hemorrhage); however, symptoms related to facial and vestibulocochlear nerve dysfunction frequently are described. At CT, AICA aneurysms are usually slightly hyperdense with strong uniform enhancement. Multidetector CT angiography depicts these lesions to excellent advantage, even when they are intracanalicular in nature. At MR imaging, a flow void is expected on T1 and T2 weighted images unless thrombus is present, which may result in variable signal intensities. Cavernous hemangiomas Cavernous hemangiomas of the IAC are rare.60,61 They manifest increased T1 signal and focal regions of decreased T2 signal. There is typically a heterogeneous enhancement pattern and regions of increased magnetic susceptibility.

Primary Parenchymal Neoplasms

Lesions of the brainstem or cerebellum rarely may have an exophytic component resulting in a CPA mass. Careful attention to all magnetic resonance pulse sequences usually will reveal an infiltrative intra-axial component that clarifies the diagnosis. Childhood neoplasms such as medulloblastoma, astrocytoma, and ependymoma may present in this fashion. Astrocytoma is the most common lesion to result in this phenomenon in the adult patient.

Choroid Plexus Papilloma

The choroid plexus courses into the lateral recess of the fourth ventricle by means of the foramen of Luschka and extends as far laterally as the CPA; therefore, choroid plexus papilloma must be kept in mind when a CPA mass is identified. Intense homogeneous enhancement with iodinated contrast (CT) and gadolinium (MR imaging) is the rule.54

ADDITIONAL DIFFERENTIAL DIAGNOSTIC ENTITIES Vascular

Vascular loops Surgical decompression of a vascular loop is often useful in the treatment of unremitting facial pain (fifth nerve) and hemifacial spasm (seventh nerve). There is, however, considerable debate as to whether vascular loops can produce auditory or vestibular symptoms.5557 A recent study attempted to correlate hearing loss with AICA loops.55 The studys authors used the following definitions:

Fig. 18. Vascular loop, hearing loss, and vertigo. (A) Magnetic resonance angiography source image reveals that the loop (white arrows) extends into the canal as far as the fundus. (B) Conventional angiogram confirms an anterior inferior cerebellar artery loop (white arrows). (Courtesy of C.D. Phillips, MD, Charlottesville, VA.)

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Fig. 19. Aneurysm in the cerebellopontine angle. (A) Axial T2W fast-spin-echo image reveals a cerebellopontine angle mass containing regions of heterogeneous T2 hyposignal, indicating the presence of hemorrhage in this partially thrombotic aneurysm. (B) The axial source image reveals T1 hypersignal. (C) Corresponding drawing depicts an aneurysm displacing the neural structures. (Courtesy of Amirsys, Inc., Salt Lake City, UT; with permission.)

Multiple cavernoma syndrome is a familial process that can present in an autosomal dominant fashion. Vascular malformations Areteriovenous malformations characterized by enlarged intensely enhancing vessels are rare in the CPA. As with other cerebral AVMs, angiography will demonstrate the feeding and draining vessels best and determine the best therapeutic management. An important differential is the acquired dural arteriovenous fistula with venous varix. Ossifying hemangioma Ossifying hemangioma is an uncommon but wellknown temporal bone lesion, typically originating along the facial nerve canal, particularly at the first genu.62 This lesion is characterized by intense contrast enhancement and bony spiculations

similar to hemangiomas noted elsewhere. Hemangiomas of the IAC tend to be more symptomatic than an acoustic schwannoma of equal size (Fig. 20). Differentiation from meningioma may be difficult.

Non-Neoplastic Meningeal Disease

Overview The meninges are the dura, dense connective tissue attached to the calvarium, the pia, a membrane firmly attached to the surface of the brain, and the arachnoid, which is a complex network of reticular fibers interposed between the pia and the dura.50 Importantly, the pia and arachnoid collectively are referred to as the leptomeninges; the dura is referred to as the pachymeninges. The leptomeninges is contiguous with the vestibulocochlear nerve. Any leptomeningeal disease process may manifest within the IAC and cause

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Fig. 20. Ossifying hemangioma, intracanalicular. Axial (A) and coronal (B) CT images reveal an eccentric expansion within the fundus of the internal auditory canal. There are calcifications within the lesion (black arrows in A), and a generalized hyperdensity is noted in (B). These findings are highly suggestive of an ossifying hemangioma. Axial precontrast (C) and postcontrast (D) images reveal intense contrast enhancement. (From Swartz JD. Lesions of the cerebellopontine angle and internal auditory canal: diagnosis and differential diagnosis. Semin Ultrasound CT MR 2004;25(4):33252; with permission.)

vertigo or retrocochlear hearing loss. Pathologic enhancement within the CPA and IAC may occur as a consequence of the entire gamut of meningeal disease. There is extensive overlap in the imaging appearance of benign and malignant disease. Meningitis Meningitis may result in pathologic leptomeningeal enhancement identical to that caused by neoplasm and commonly involves the IAC/CPA. Bacterial and fungal diseases are more predisposed to this manifestation than viral disease.63 Neurosarcoidosis Neurosarcoidosis occurs in up to 25% of patients who have systemic sarcoidosis and typically manifests as leptomeningeal thickening and enhancement (Fig. 21). The leptomeninges are involved somewhat more commonly than the pachymeniniges. This granulomatous process may result in both diffuse and nodular enhancement patterns.

IAC/CPA involvement is not uncommon and is often bilateral, perhaps masquerading as NF-2. This often results in retrocochlear sensorineural hearing loss.9 Involvement of other cranial nerves is also very common. Additional conditions Pathologic enhancement of the meninges may result from various additional conditions. Pachymeningeal enhancement routinely is seen in postcraniotomy patients. On occasion, such enhancement is extensive and quite thick and may persist for several years. Intracranial hypotension may be spontaneous or result from lumbar puncture or head trauma.64 The spontaneous variety (SIHH) is associated with a distinctive postural headache. Occult CSF leak is the presumed cause. The meningeal enhancement associated with this condition is often quite extensive and is most commonly pachymeningeal but may involve the leptomeninges including the IAC (Fig. 22).

Pathology of the Vestibulocochlear Nerve

Fig. 21. Neurosarcoidosis. Axial (A) and coronal (B) enhanced T1W image reveals a cerebellopontine angle mass and nodules of enhancement in the adjacent leptomeninges (black arrows) and pachymeninges (white arrows). (From Swartz JD. Lesions of the cerebellopontine angle and internal auditory canal: diagnosis and differential diagnosis. Semin Ultrasound CT MR 2004;25(4):33252; with permission.)

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Miscellaneous Conditions
Siderosis Superficial siderosis is characterized by deposition of hemosiderin in the leptomeninges resulting from subarachnoid hemorrhage that overwhelms the ability of the blood stream to remove the hemosiderin-laden macrophages.65,66 Hearing impairment is an important feature, and its absence is exceptional.66 The vestibulocochlear nerve (VCN) is vulnerable to hemosiderin for several reasons. The VCN is lined extensively with central myelin, which is supported by microglia. These microglia are uniquely sensitive to iron-mediated cell damage. Furthermore, the VCN has a long and tortuous

course and as such is exposed to substantial CSF flow. The olfactory tract and bulb are also vulnerable because of their similar anatomy.66 MR imaging findings of siderosis are pathognomonic. T2 weighted and, in particular, gradient echo susceptibility imaging reveals characteristic hypointensity most easily appreciated along the surface of the brain stem and cerebellar vermis (Fig. 23). The multiple refocusing 180 pulses of FSE imaging may make the hemosiderin less apparent as they reduce the T2* effect, and low field magnets are also less susceptible to the paramagnetic effects of hemosiderin. Gradient echo imaging is the ideal sequence as it results in blooming of the T2* effect.

Fig. 22. Intracranial hypotension in a 34-year-old patient with postural headaches. (A) Coronal T2W image reveals T2 hypersignal reflecting thickening of the pachymeninges (white arrows). (B) Enhanced coronal T1W image confirms pathologic thickening and enhancement along the tentorium, cerebellopontine angles, and the cervical epidural space (white arrows).

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Fig. 23. Siderosis. Axial (A) and coronal (B) T2W images reveal extensive T2 hyposignal along the surface of the brainstem and temporal lobes, reflecting increased magnetic susceptibility caused by hemosiderin deposition.

The extent of siderosis does not necessarily correlate with the severity of clinical disease.65,66 Some patients who have extensive imaging evidence have minimal symptoms, whereas others with less severe findings may be debilitated significantly. Labyrinthitis Labyrinthitis refers to inflammatory disease of the perilymphatic spaces of the inner ear, which results in secondary changes within the endolymphatic spaces (membranous labyrinth).67 The most common symptoms are sensorineural hearing loss and vertigo, which may be recurrent or debilitating. Tympanogenic labyrinthitis is typically unilateral and secondary to middle ear disease. Meningogenic labyrinthitis is usually bilateral and occurs secondary to meningitis. The latter probably results from spread by means of the fundus of the IAC into the vestibule or by means of the cochlear nerve foramen into the cochlear apex with internal

dissemination via the modiolus.68 Propagation by means of the cochlear aqueduct is probably less common. Meningogenic labyrinthitis typically occurs in children and is the most common cause of acquired childhood deafness.69 Labyrinthitis may be viral (most common) as well as autoimmune, bacterial or luetic. Otosyphilis also may manifest as an osteitis, resulting in otic capsule demineralization or a meningitis involving the IAC.70 The classic imaging finding in acute/subacute labyrinthitis is enhancement of the normally nonenhancing fluid-filled spaces of the labyrinth as seen on contrast-enhanced T1 weighted images (Fig. 24).71 The reader should be aware that most patients diagnosed with viral labyrinthitis will not have labyrinthine enhancement (or any other imaging finding). If acute labyrinthitis does not resolve, a progression to chronic disease initially results in fibrous changes followed by ossification. The fibrous stage of this disorder manifests as T2 hyposignal on MR

Fig. 24. Viral labyrinthitis. Recent onset of hearing loss and vertigo in a 47-year-old man. (A) Precontrast coronal T1W image of the left ear. (B) Postcontrast coronal T1W image of the left ear. The normally fluid-filled spaces of the anterior cochlear turns enhance intensely after contrast administration (white arrows).

Pathology of the Vestibulocochlear Nerve

imaging and a normal CT. The ossific stage is characterized by labyrinthine ossification at CT that often is profound (see Fig. 3). MR imaging is also abnormal at this stage. Intermediate stages also exist. Trauma Hearing loss is a common sequela of temporal bone injury. SNHL may of course result from fracture through the IAC or bony labyrinth; however, it may also develop because of a severe concussive force (labyrinthine concussion) in the absence of demonstrable fracture.67 There are no CT findings, and MR imaging is negative unless intralabyrinthine hemorrhage is present. SNHL also could result from brain stem contusion at the level of the upper medulla. Shearing force at the root entry zone is an additional cause. Rarely, contusions of the inferior colliculi may be diagnosed consequent to the traumatic event. Impaction of tentorium against the dorsal midbrain secondary to accelerationdeceleration forces is the presumed cause. Delayed or fluctuating SNHL associated with persistent vertigo may result from a perilymphatic fistula, an aberrant perilymphatic leakage into the middle ear. Intralabyrinthine hemorrhage Imaging documentation of intralabyrinthine hemorrhage (ILH) is rare.67,72,73 Noncontrast thinsection T1 weighted images easily demonstrate the bright signal from the methemoglobin in distinct contrast to the low signal/signal void of surrounding bone. The ability to make this diagnosis mandates the need for precontrast thin-section T1 weighted images (Fig. 25). There are numerous potential causes of ILH, and there have been numerous individual case reports documenting this process in various disease states. ILH can be secondary to coagulopathy, tumor, trauma, and sickle cell disease.

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AUDITORY PATHWAY
The hair cells of the organ of Corti subtend the neurons of the spiral (cochlear) ganglion, which reside within the modiolus and give origin to the cochlear nerve. The cochlear nerve travels through the cochlear nerve aperture at the apex of the cochlea and subsequently along the anteroinferior quadrant of the internal auditory canal and through the cerebellopontine angle cistern to the dorsal and ventral cochlear nuclei that lie along the lateral surface of the inferior cerebellar peduncle at the rostral pole of the upper medulla.69,74 Fibers from the cochlear nuclei course superiorly to the lateral lemniscus and subsequently to the inferior colliculus in the midbrain where they synapse. The auditory pathway continues to the medial geniculate body of the thalamus and subsequently to the auditory cortex in the temporal lobe. There are a few collaterals from the cochlear nuclei that course to the superior olive and then cross in the pons through the trapezoid body. Lesions involving the cochlear nuclei in the medulla result in unilateral retrocochlear hearing loss identical to that caused by compression of the cochlear nerve (Figs. 26 and 27). Avoidance of internal auditory canal tunnel vision is paramount when evaluating these patients. Lesions involving the more peripheral auditory pathway result in less severe and bilateral deficits or perhaps even an auditory agnosia.74 This is defined as

Fig. 25. Intralabyrinthine hemmorhage. Noncontrast T1W image reveals pathologic hypersignal throughout the cochlea and vestibule (white arrows) in a trauma patient. (Courtesy of Jan W. Casselman, MD, PhD, Bruges, Belgium.)

Fig. 26. Acute disseminated encephalomyelitis (ADEM). This patient suffered hearing loss following a vaccination. There is a hyperintense lesion within the posterolateral aspect of the upper medulla on the left (white arrow) in the vicinity of the cochlear and vestibular nuclei. Differential diagnosis includes demyelinating disease (in this case monophasic) and ischemic disease. (Courtesy of C.D. Phillips, MD, Charlottesville, VA.)

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Fig. 27. Multiple sclerosis, retrocochlear hearing loss. (A) Axial T2W images; there is a focus of hypersignal within the posterolateral part of the upper medulla (outlined black arrow) in a patient with demyelinating disease. (B) Artists rendering; note corresponding location of cochlear nuclei. (From Swartz JD, Daniels DL, Harnsberger HR, et al. Hearing, II: the retrocochlear auditory pathway. AJNR Am J Neuroradiol 1996;17:147981; with permission.)

preservation of the sensation of hearing but impaired recognition of sound and words.75 Intra-axial lesions responsible for SNHL can be ischemic, demyelinating, traumatic, vascular, and neoplastic. The most common are infarcts and demyelinating disease (multiple sclerosis).

COCHLEAR IMPLANT SURGERY

Components of the Cochlear implant include a speech processor, a headset and microphone, a receiver and stimulator, and an intracochlear electrode array responsible for stimulating the

Fig. 28. Cochlear implant. (A) Axial CT image demonstrates an electrode array at the site of introduction by means of a cochleostomy (black arrow) in the vicinity of the round window. (B) Axial CT image through the cochlear apex indicates that the device (black arrows) is well-positioned in the middle and basal cochlear turn. (C) Artists rendering of the device.

Pathology of the Vestibulocochlear Nerve

cochlear nerve.76 The receiver/stimulator is placed beneath the postauricular soft tissues within the calvarium. The electrode array is inserted for a variable distance by means of the round window (or anteroinferior basilar turn fenestration cochleostomy) into the scala tympani of the basilar turn (Fig. 28). The interscalar septum and the basilar membrane provide protection for the scala media (cochlear duct). Scala vestibuli insertions are much more likely to cause damage to Reissners membrane and residual hair cells. An attempt is made to replicate the tonotopic organization of the cochlea by orienting the electrode to specific electrode contacts along the electrode array. The electrical stimulation that corresponds with the highest pitches is delivered within the basilar region of the cochlea, and the lowest pitches are delivered toward the apical region of the cochlea consistent with normal physiology. Auditory brainstem implants (ABI) are used at some institutions. They are placed in the lateral recess of the fourth ventricle and allow for direct stimulation of the cochlear nuclei in the medulla in individuals with nonfunctioning or absent cochlear nerves. The flat rectangular shape of the stimulating array facilitates placement in the foramen of Luschka. An ABI is similar to a cochlear implant in the way it receives and processes sound.77,78 Preoperative CT without contrast is recommended for all cochlear implant candidates79 MR imaging may be of significant value also. Analysis of these images not only aids in determining the likelihood of success of the procedure, but also influences the choice of implant and allows the surgeon to choose the best side for implantation. Ossification obliterates the membranous labyrinth and potentially limits cochlear patency. When extensive, such ossification may effectively preclude implantation in many patients. Preoperative imaging in individuals who have diffuse inner ear malformation also must focus on the potential for perilymph gusher following insertion of the electrode.80 Specifically the images must evaluate the anatomy of the fundus of the IAC and the base of the cochlea. The number of contraindications to cochlear implant is diminishing rapidly as the technology and surgical skill increase.81,82 The lone complete contraindication is an absent cochlear nerve, which may be congenital or acquired.1 Plain film (radiograph) provides an excellent overview and assessment of insertion depth of the device.83 A modified Stenvers view has been suggested for this purpose. In difficult cases, postoperative CT has been advanced to provide an assessment of the intracochlear position of the electrode array.84 The precise location of each electrode contact can be assessed in this manner. Successful CI requires that the electrodes be restricted to the scala tympani, and CT is useful in this regard. The presence of a functioning cochlear implant was a contraindication to MR imaging examination. This is unfortunate, as many of these patients may develop conditions for which MR imaging may be a useful diagnostic procedure. Recently one cochlear implant manufacturer officially announced that their implant is compatible with MR imaging at or below 1.5 Tesla. Some precautions have to be made, and a tape has to be tightened around to head to keep the magnet in place. Unfortunately many other companies did not announce magnetic resonance compatibility yet. Hence magnetic resonance compatibility has to be checked in every case before MR imaging.

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conventional spin-echo MR? AJNR Am J Neuroradiol 1996;17:120510. Xenellis JE, Linthicum FH. On the myth of the glial/ schwann junction (Obersteiner-Redlich zone): origin of vestibular nerve schwannomas. Otol Neurotol 2003;24:1. Tali ET, Yuh WTC, Nguyen HD, et al. Cystic acoustic schwannomas: MR characteristics. AJNR Am J Neuroradiol 1993;14:12417. Spickler EM, Govila L. The vestibulocochlear nerve. Semin Ultrasound CT MR 2002;23:21837. Ishikawa K, Okamoto K, Furusawa T, et al. Inner ear signals on mr cisternography: clues to differentiate between acoustic neurinomas and cerebellopontine angle meningiomas. Presented at the Radiological Society of North America. Chicago (IL), 2006. Okamoto K, Furusawa T, Ishikawa K, et al. Focal T2 hyperintensity in the dorsal brain stem in patients with vestibular schwannoma. AJNR Am J Neuroradiol 2006;27:130711. Copelan BB, Sarver CE, Zee C, et al. Imaging of the postoperative temporal bone: an educational review. Presented at the Radiological Society of North America. Chicago (IL), 2006. Warren FM, Kaylie DM, Aulino JM, et al. Magnetic resonance appearance of the inner ear after hearingpreservation surgery. Otol Neurotol 2006;27:3937. Pollock BE. Management of vestibular schwannomas that enlarge after stereotactic radiosurgery: treatment recommendations based on a 15-year experience. Neurosurgery 2006;58:2418. Weissman JL, Hirsch BE, Fukui MB, et al. The evolving MR appearance of structures in the internal auditory canal after removal of an acoustic neuroma. AJNR Am J Neuroradiol. 1997;18:31323. Smith M, Castillo M, Campbell J, et al. Baseline and follow-up MRI of the internal auditory canal after suboccipital resection of acoustic schwannoma: appearances and clinical correlations. Neuroradiology 1995;37:31720. Kremer P, Forsting M, Hamer J, et al. MR enhancement of the internal auditory canal induced by tissue implant after resection of acoustic neurinoma. AJNR Am J Neuroradiol 1998;19:1158. Thedinger BA, Glasscock ME, Cueva RA, et al. Postoperative radiographic evaluation after acoustic neuroma and glomus jugulare removal. Laryngoscope 1992;102:2616. Larson TL. Understanding the post-treatment appearance of the internal auditory canal and cerebellopontine angle. Semin Ultrasound CT MR 2003;24:13346. Brors D, Schafers M, Bodmer D, et al. Postoperative magnetic resonance imaging findings after transtemporal and translabyrinthine vestibular schwannoma resection. Laryngoscope 2003;113:4206. Tieleman A, Casselman JW, Somers T, et al. Imaging of intralabyrinthine schwannomas: a retrospective study of 52 cases with emphasis on lesion growth. AJNR Am J Neuroradiol, in press. Kennedy RJ, Shelton C, Salzman KL, et al. Intralabyrinthine schwannomas: diagnosis, management, and a new classification system. Otol Neurotol 2004;25:1607. Hegarty JL, Patel S, Fischbein N, et al. The value of enhanced magnetic resonance imaging in the evaluation of endocochlear disease. Laryngoscope 2002;112:817. Aho TR, Daspit CP, Dean BL, et al. Intralabyrinthine meningioma. AJNR Am J Neuroradiol 2003;24: 16425. Hamilton BE, Salzman KL, Patel N, et al. Imaging and clinical characteristics of temporal bone meningioma. AJNR Am J Neuroradiol 2006;27:22049. Bacciu A, Piazza P, Di Lella F, et al. Intracanalicular meningioma: clinical features, radiologic findings, and surgical management. Otol Neurotol 2007;28: 3919. Dahlen RT, Johnson CE, Harnsberger HR, et al. MR imaging characteristics of intravestibular lipoma. AJNR Am J Neuroradiol 2002;23:14137. Bonneville F, Sarrazin J, Marsot-Dupuch K, et al. Unusual lesions of the cerebellopontine angle: a segmental approach. Radiographics 2001;21:41938. Kertesz TR, Shelton C, Wiggins RH, et al. Intratemporal facial nerve neuroma: anatomical location and radiological features. Laryngoscope 2001;111: 12506. Stasolla A, Bellussi A, Bibbolino C. Dural tail: another face of facial nerve schwannoma? AJNR Am J Neuroradiol 2006;27:18047. Rokni-Yazdi H, Sotoudeh H. Prevalence of dural tail sign in patients with different intracranial pathologies. Eur J Radiol 2006;60:425. Oner AY, Tokgoz N, Tali ET, et al. Imaging meningiomas: is there a need for postcontrast FLAIR? Clin Radiol 2005;60:13005. Martinez R, Vaquero J, Areitio E, et al. Meningiomas of the posterior fossa. Surg Neurol 1983;19:23743. Asaoka K, Barrs DM, Sampson JH, et al. Intracanalicular meningioma mimicking vestibular schwannoma. AJNR Am J Neuroradiol 2002;23:14936. Magliulo G, Zardo F, Bertin S, et al. Meningiomas of the internal auditory canal: two case reports. Skull Base 2002;12:1926. Roche PH, Regis J. Cerebellopontine angle meningiomas. J Neurosurg 2005;103:9357, author reply 9378. Ben Hamouda M, Drissi C, Sebai R, et al. Atypical CT and MRI aspects of an epidermoid cyst. J Neuroradiol 2007;34:12932. Eldevik OP, Blaivas M, Gabrielsen TO, et al. Craniopharyngioma: radiologic and histologic findings and recurrence. AJNR Am J Neuroradiol 1996;17: 142739.

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